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@ARTICLE{Castelli:309671,
author = {S. Castelli and F. Schulze and T. M. Thole-Kliesch and K.
Astrahantseff and G. Barone and M. Beck-Popovic and P.
Berlanga and S. Corbacioglu and M. Fischer and M. Gambart
and S. L. George and L. Chesler and J. C. Gray and B. Hero
and A. Künkele$^*$ and T. Flaadt and P. Lang and H. N. Lode
and J. J. Molenaar and G. Schleiermacher and C. Rosswog and
L. Moreno and C. Owens and A. Rubio-San-Simón and J.
Schulte$^*$ and T. Simon and D. A. Tweddle and H. E.
Deubzer$^*$ and A. Eggert$^*$ and I. SIOPEN New Drug
Development},
collaboration = {R. Groups},
title = {{C}urrent treatment strategies for first relapse of
high-risk neuroblastoma.},
journal = {European journal of cancer},
volume = {236},
issn = {0959-8049},
address = {Amsterdam [u.a.]},
publisher = {Elsevier},
reportid = {DKFZ-2026-00293},
pages = {116254},
year = {2026},
note = {#DKTKZFB26#},
abstract = {More than 50 $\%$ of patients with high-risk neuroblastoma
(HRNB) will relapse despite intensive multimodal therapy.
Most relapses occur within 2 years of diagnosis. Overall
survival at relapse is 20 $\%$ at 4 years, but long-term
survival can be achieved in a patient subset. A biopsy at
relapse with in-depth molecular characterization should now
become accepted as standard of care to confirm active
neuroblastoma and identify potential targets for
biomarker-based targeted therapy or immunotherapy. No clear
consensus currently exists about optimal therapy because the
field lacks umbrella trials covering all phases of relapse
treatment (re-induction, consolidation, maintenance) in a
homogenous strategy. Recruitment into clinical trials (e.g.
BEACON2) should be prioritized. Current evidence supports
starting re-induction therapy with a camptothecin-based
chemotherapy regimen combined with monoclonal antibody
therapy targeting GD2 or VEGF (or ALK inhibitors if
ALK-aberrant) as the first choice. The RIST regimen is a
promising first choice for MYCN-amplified disease. After an
objective response to re-induction therapy, GD2-directed
immunotherapy or cellular therapies harnessing the immune
system (haploidentical stem cell transplantation, CAR T
cells) are of high interest as a consolidation strategy.
Long-term maintenance therapy must be feasible as outpatient
treatment, have a low toxicity profile and be well-tolerable
to suit patients with relapsed HRNB. For optimal care, new
options must be tested as maintenance therapy in randomized
trials. The most promising salvage options for patients
responding insufficiently to treatment are the chemotherapy
combinations, topotecan/vincristine/doxorubicin (TVD),
topotecan/cyclophosphamide/etoposide (TCE),
ifosfamide/carboplatin/etoposide (ICE) or
topotecan/cyclophosphamide (TopoCy), or [131I]-mIBG therapy.
Early-phase clinical trials are also a possible option in
this setting.},
subtyp = {Review Article},
keywords = {Anaplastic lymphoma kinase (Other) / CAR T cells (Other) /
Camptothecins (Other) / Chemoimmunotherapy (Other) /
Disialoganglioside GD2 (Other) / Embryonal tumor (Other) /
Haplo-SCT (Other) / Molecular tumor board (Other) /
Pediatric cancer (Other) / Precision medicine (Other)},
cin = {BE01 / TU01},
ddc = {610},
cid = {I:(DE-He78)BE01-20160331 / I:(DE-He78)TU01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41643518},
doi = {10.1016/j.ejca.2026.116254},
url = {https://inrepo02.dkfz.de/record/309671},
}